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Biomedical subjects

B B Gay

Publications and source records attributed to B B Gay.

At least 19 recordsLinked to original sources

Standard method of diagnosis versus use of a computer database in the evaluation of skeletal dysplasias.

OBJECTIVE: The objective of this study was to compare reference textbooks and the computer database, OSSUM, for accuracy and ease of use in the diagnosis of skeletal dysplasias. Materials and methods. Twenty cases of clinically and and radiologically established skeletal dysplasias were evaluated as unknowns by four pediatric radiologists. Readers 1 and 2 evaluated group A (10 cases) using reference texts and group B (10 cases) using OSSUM. Readers 3 and 4 evaluated group B using reference texts. The radiologists independently listed their roentgenographic findings, the top three diagnoses, confidence level, difficulty level, and time spent on each case. RESULTS: The correct diagnosis was made in 68% of both the reference text cases and the OSSUM cases. Difficulty level was significantly higher (3.5 vs 2.9, P = 0.0013) and confidence significantly lower (3.3 vs. 2.3, P = 0.0001) when using OSSUM. Average time spent on cases was 25 min with references and 30 min with OSSUM (P > 0.05). However, there was a decrease in both the time (38 min vs 23 min, P = 0.05) and the difficulty (3.9 vs 3.1, P = 0.001) between the first five and the last five cases. The composite of four readers correctly identified 90% of the skeletal dysplasias when the results of both methods were combined. CONCLUSIONS: In the ability to reach a correct diagnosis, no difference was detected between the OSSUM and reference texts methods. The increased time necessary, greater difficulty and decreased confidence levels with OSSUM are expected to improve with increasing program familiarity. Use of both textbooks and the database was complementary.

Bone Diseases, Developmental↗

Fibromatoses of childhood: the spectrum of radiographic findings.

Fibrous tumor of childhood include several disorders with variable biologic behavior. In the review by Coffin and Dehner [1] of 190 soft-tissue neoplasms in 183 children, 27% were fibroblastic or myofibroblastic in origin. Although nearly all fibrous lesions are benign, they may be locally aggressive. The purpose of this essay is to describe clinical characteristics and to illustrate radiologic features of commonly encountered fibrous lesions of childhood. Familiarity with the presentation and variable appearance may aid the radiologist in suggesting the diagnosis of fibromatosis.

Adolescent↗

Osteopathia striata with cranial sclerosis.

Osteopathia striata with cranial sclerosis (OS-CS) is a specific bone dysplasia manifested by hypertelorism, flat nasal bridge, frontal bossing, large head, hypoplastic maxilla, palate anomalies, chronic otitis media, hearing deficits, nasal obstruction, and neurological changes of deafness, facial palsy, ophthalmoplegia, and mental retardation. We will review the clinical and radiologic findings in a new patient from birth to 20 years; this is believed to be the thirty-fifth patient reported. OS-CS is 2.5 times more common in females and occurs as an autosomal dominant condition or a sporadic dominant mutation with patients presenting for evaluation from the newborn period to the fifth decade. Skeletal abnormalities are distinctive including sclerosis of the skull base and calvarium, linear striated densities in the long bones and pelvis, and poor development of the mastoid and sinus air cells. Radionuclide bone scans with SPECT indicated in our patient increased bone turnover which was supported by biochemical findings of increased pyridinoline excretion. The major complications are due to constriction of essential foramina at the skull base. The condition is not life-threatening but can produce disability.

Adult↗

Wandering spleen: anatomic and radiologic considerations.

Wandering spleen is a rare clinical entity and remains an elusive clinical diagnosis, particularly in the pediatric patient. Among the imaging modalities (computerized tomography, magnetic resonance imaging, nuclear medicine, ultrasonography, plain films) used in the diagnosis of wandering spleen, ultrasonography is the least invasive and most effective. Elective splenopexy remains the treatment of choice when the diagnosis is made before splenic infarction. We present two cases of wandering spleen manifested as a lower abdominal mass, and we discuss the surgical anatomy of the spleen and splenic ligaments and the hypothetical responsibility of these ligaments for the genesis of this clinical entity.

Adolescent↗

Campylobacter gastritis simulating Menetrier's disease by upper gastrointestinal radiography.

Within this decade it has been determined that primary gastritis in both children and adults is frequently associated with infection of the gastric mucosa with Campylobacter pylori. It is characterized by a chronic inflammatory process in which the mucosa of the gastric antrum is typically most severely involved. Other regions of the stomach may be involved and associated peptic ulcers of the stomach and duodenal bulb are frequent. A case of C. pylori gastritis is reported in which involvement of the gastric fundus and body produced severe rugal hypertrophy that resembled Menetrier's disease.

Campylobacter Infections↗

Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice in childhood.

Idiopathic fibrosing pancreatitis is a chronic process of unknown etiology characterized by extensive infiltration of the pancreatic parenchyma by fibrous tissue. This disease process is uncommon in the pediatric patient and is consequently rarely considered in the differential diagnosis of abdominal pain and jaundice in the child. The sonographic demonstration of a dilated biliary tree and common bile duct compressed by an enlarged pancreas may be the first suggestion of this entity. Two patients with idiopathic fibrosing pancreatitis and obstructive jaundice are reported with a review of the clinical, radiographic, and pathologic findings.

Biopsy↗

Esophageal stenosis with esophageal atresia.

Esophageal atresia with tracheosophageal fistula may be associated rarely with distal esophageal stenosis. Three patients are reported with this combination of esophageal anomalies. In addition the clinical and radiologic features of 24 patients previously reported in the literature are reviewed. Careful evaluation of the distal esophagus during postoperative contrast studies in patients with esophageal atresia should be obtained to exclude distal stenosis. The presence of unrecognized distal esophageal stenosis may lead to complications of postoperative anastomotic leaks, poor healing of the anastomosis, aspiration, and impaction of a solid food bolus proximal to the stenosis.

Esophageal Atresia↗

Subglottic foreign bodies in pediatric patients.

Impacted subglottic foreign bodies may produce upper airway obstruction and clinical signs simulating croup or asthma. We identified the roentgenologic and clinical features in six patients. In four of these patients, the parent had not observed the aspiration episode, so that the diagnosis was delayed. Roentgenologic studies demonstrated subglottic narrowing of the upper airway with a homogeneous, poorly defined radiodensity within the narrowed segment. These roentgenologic studies are usually diagnostic; therefore, if infants or young children present with stridor of undetermined cause, soft-tissue upper airway roentgenography is indicated.

Asthma↗

Klippel-Trenaunay and Sturge-Weber syndromes with renal hemangioma and double inferior vena cava.

We describe a 3 1/2-year-old boy with the Klippel-Trenaunay and Sturge-Weber syndromes. The child had congenital superficial capillary hemangiomas, congenital glaucoma and mild hydrocephalus. During the first year of life he experienced intermittent hematuria. When he was 3 years old he presented with seizures and left hemihypertrophy first was noted. Several months later radiological examination of a large abdominal mass demonstrated its origin to be in the right kidney. Radical nephrectomy documented the presence of renal hemangioma with complicating perirenal hematoma. A double inferior vena cava was another unexpected surgical finding that complicated the course of this patient. All of these unusual features in these rare syndromes with their clinical, pathogenetic and therapeutic implications are discussed. The differential diagnosis of renal masses in these syndromes also is presented.

Angiomatosis↗

Cystic neuroblastoma in infants: radiographic and pathologic features.

Cystic neuroblastoma is a rare form of neuroblastoma. Three cases of cystic neuroblastoma in the infant are reported with emphasis on the sonographic findings. In two cases, the tumor was demonstrated in the fetus. The pathologic features of the tumors are described. Development of cysts may be related to a prominent microcystic arrangement of tumor nests.

Adrenal Gland Neoplasms↗

Characteristics of children with endoscopically proved chronic bronchitis.

This study evaluated by chart review the clinical, allergic, pathologic, and immunologic characteristics of 20 children found to have chronic bronchitis (CB) by bronchoscopic evaluation, including 13 children in whom CB was documented by endobronchial biopsy. Two additional children likely to have had CB also are described. In this study group, all of the patients were believed to have asthma as well. Chronic bronchitis was predicted by chronicity of symptoms and incomplete response to bronchodilators and corticosteroids, but it was not predicted by a history of allergy or laboratory evidence of systemic infection or inflammation. In the children described herein, CB often was associated with an IgG subclass abnormality. Bronchoscopic evaluation documented CB and provided biopsy and secretion samples. Although the histomorphic findings were heterogeneous, patients with CB usually had white blood cells present on Gram stains of secretions in addition to mononuclear cell infiltrates on biopsy specimens. Chronic bronchitis in these children seems to be distinct from CB in adults. Further studies will be needed to define CB in children.

Adolescent↗

Adrenal abscess in the neonate.

Adrenal abscess in the neonate is a rare complication of adrenal hemorrhage. The radiographic and clinical findings of 12 previously published cases and two new cases of adrenal abscess in the newborn are presented. Sonography was the most helpful examination in distinguishing a suprarenal lesion from an intrarenal lesion and in demonstrating the morphology of the abscess.

Abscess↗

Wilms tumor in horseshoe kidneys: radiologic diagnosis.

Two cases of nephroblastoma occurring in a horseshoe kidney are reported, and 32 cases from the literature are reviewed. The radiologic signs of horseshoe kidney may be difficult to evaluate with excretory urography when the mass is large. Rotational abnormalities of the opposite kidney that is not involved by tumor should suggest the possibility of an associated horseshoe kidney. Real-time ultrasonography and computed tomography are helpful in identifying the isthmus of the horseshoe kidney. Aortography confirms the presence of the horseshoe kidney and demonstrates the arterial supply to the isthmus and the tumor. Radionuclide scans demonstrate the isthmus when the tumor arises from an upper pole, but may not be diagnostic if the tumor arises from the isthmus.

Aortography↗

Ultrasound diagnosis of hypertrophic pyloric stenosis: real-time application and the demonstration of a new sonographic sign.

The diagnosis of hypertrophic pyloric stenosis (HPS), a common problem of infancy, is usually based on medical history. When the diagnosis is in question a barium upper gastrointestinal series has been the diagnostic imaging procedure of choice. In this study real-time ultrasound was used in 27 infants in whom pyloric stenosis was suspected. The published criteria for the ultrasound diagnosis of pyloric stenosis were used to evaluate the results. Fourteen examinations were true-positive, 12 were true-negative, and one was false-negative. The authors termed the hypertrophied muscle, which was demonstrated in longitudinal section, the "ultrasonic cervix sign" of hypertrophic pyloric stenosis. It was concluded that real-time ultrasound is a simple and accurate method for the diagnosis of HPS and should be the initial imaging procedure.

Evaluation Studies as Topic↗

Choledochal cysts in children: radiologic features.

Choledochal cysts, although uncommon, are found most frequently in children. Radiologic studies have always played an important role in diagnosis because of the nonspecific clinical features. The abdominal roentgenogram may show in the right upper abdomen a mass, the cystic nature of which can be demonstrated by intravenous urography, hepatic angiography, sonography, and computerized tomography. Large choledochal cysts will displace the duodenal loop inferiorly and to the left, as seen on upper gastrointestinal contrast studies. Oral cholecystography and intravenous cholangiography are currently not indicated because of the poor diagnostic yield and the significant toxicity of intravenous media in children. Operative cholangiography is desired by the surgeon for demonstration of the pathologic anatomy. Real-time sonography and cholescintigraphy with technetium Tc 99m iminodiacetic acid derivative allow specific preoperative diagnosis and will eliminate the need for more invasive studies, such as arteriography, endoscopic cholangiopancreatography, and percutaneous transhepatic cholangiography. The radiologic findings in six patients are summarized.

Angiography↗